Cerebral dural arteriovenous fistulas in patients with PTEN-related hamartoma tumor syndrome.

Central nervous system (CNS) dural arteriovenous fistulas (DAVF) have been reported in PTEN-related hamartoma tumor syndrome (PHTS). However, PHTS-associated DAVF remain an underexplored field of the PHTS clinical landscape. Here, we studied cases with a PTEN pathogenic variant identified between 2007 and 2020 in our laboratory (n = 58), and for whom brain imaging was available. Two patients had DAVF (2/58, 3.4%), both presenting at advanced stages: a 34-year-old man with a left lateral sinus DAVF at immediate risk of hemorrhage, and a 21-year-old woman with acute intracranial hypertension due to a torcular DAVF. Interestingly, not all patients had 3D TOF/MRA, the optimal sequences to detect DAVF. Early diagnosis of DAVF can be lifesaving, and is easier to treat compared to developed, proliferative, or complex lesions. As a result, one should consider brain MRI with 3D TOF/MRA in PHTS patients at genetic diagnosis, with subsequent surveillance on a case-by-case basis.

Natural history and management of spinal cord arteriovenous shunts in pregnancy: A monocentric series of 10 consecutive cases with emphasis on endovascular treatment.

PURPOSE: Intradural spinal cord arteriovenous shunts (IDSCAVS) are rare and constitute a challenging situation if symptoms occur during pregnancy. We present a series of ten such cases referred to our center: five cervical, four thoracic and one lumbar. METHODS: We retrospectively reviewed our global series of 215 IDSCAVSs between 2002 and March 2020 and found ten patients who had presented during pregnancy. Clinical, radiological and therapeutic data were studied. RESULTS: Seven shunts were AVM type niduses and three were micro AV-fistulae. All were associated with pial venous reflux and six hemorrhagic cases had pseudo aneurysms. Symptoms occurred mainly during the third trimester, 80% of patients presented with hemorrhage and spinal cord dysfunction. We embolized seven patients and proposed surgery in one, always after delivery: all recovered well. One woman declined treatment; one other was operated in emergency but did not improve. Mean follow-up was 3.9 years (0.5...19 years). CONCLUSIONS: Despite this small group of patients, our initial experience of IDSCAVSs diagnosed during pregnancy indicates that embolization is an effective management strategy if performed after delivery and a recovery period. Results indicate that IDSCAVSs seem to have a low risk of early rebleedings after the ictal event and may be closely followed up until delivery. The results obtained show good clinical outcome without long-term rebleeds. Women with known IDSCAVSs should not be discouraged from becoming pregnant, however it seems wise to embolize them before pregnancy in order to offer protection against risks during pregnancy.

A comparison of digital subtraction angiography and computed tomography angiography for the diagnosis of penetrating cerebrovascular injury.

OBJECTIVE: Penetrating cerebrovascular injury (PCVI) is a subset of traumatic brain injury (TBI) comprising a broad spectrum of cerebrovascular pathology, including traumatic pseudoaneurysms, direct arterial injury, venous sinus stenosis or occlusion, and traumatic dural arteriovenous fistulas. These can result in immediate or delayed vascular injury and consequent neurological morbidity. Current TBI guidelines recommend cerebrovascular imaging for detection, but there is no consensus on the optimum modality. The aim of this retrospective cohort study was to compare CT angiography (CTA) and digital subtraction angiography (DSA) for the diagnosis of PCVI. METHODS: The records of all patients presenting to two level I trauma centers in the United States between January 2010 and July 2016 with penetrating head or neck trauma were reviewed. Only those who had undergone both CTA and DSA were included. Clinical and neuroimaging data were collected, and PCVIs were stratified using a modified Biffl grading scheme. DSA and CTA results were then compared. RESULTS: Of 312 patients with penetrating trauma over the study period, 56 patients (91% male, mean age 32 years) with PCVI met inclusion criteria and constituted the study cohort. The mechanism of injury was a gunshot wound in 86% (48/56) of patients. Twenty-four (43%) patients had sustained an angiographically confirmed arterial or venous injury. Compared with DSA as the gold standard, CTA had a sensitivity and specificity of 72% and 63%, respectively, for identifying PCVI. CTA had a positive predictive value of 61% and negative predictive value of 70%. Seven patients (13%) required immediate endovascular treatment of PCVI; in 3 (43%) of these patients, the injury was not identified on CTA. Twenty-two patients (39%) underwent delayed DSA an average of 25 days after injury; 2 (9%) of these patients were found to harbor new pathological conditions requiring treatment. CONCLUSIONS: In this retrospective analysis of PCVI at two large trauma centers, CTA demonstrated low sensitivity, specificity, and positive and negative predictive values for the diagnosis of PCVI. These findings suggest that DSA provides better accuracy than CTA in the diagnosis of both immediate and delayed PCVI and should be considered for patients experiencing penetrating head or neck trauma.

Intracranial dural arteriovenous fistula mistaken as cervical transverse myelitis.

We describe a man with an intracranial dural arteriovenous fistula that presented as a subacute longitudinally extensive cervical myelopathy. The uncommon location of the fistula and the absence of specific radiological signs resulted in initial misdiagnosis as longitudinally extensive transverse myelitis. Neurologists should have a high index of suspicion for dural arteriovenous fistula in older men, especially those with subacute or chronic symptoms, acellular cerebrospinal fluid and, particularly, if there is neurological deterioration soon after corticosteroid treatment. Patients need early angiography to identify this treatable cause of myelopathy.

Surgical ligation of spinal dural arteriovenous fistula.

BACKGROUND: Spinal dural arteriovenous fistulas (SDAVFs) are abnormal arteriovenous shunts between a radicular artery and the radicular vein, located in the dorsal surface of the dura sleeve, which drains in a retrograde manner into the coronal venous plexus of the spinal cord without an interposed capillary network. This result is a venous hypertension that reduces spinal cord perfusion and leads to ischemia and edema. Spontaneous resolution is extremely rare and, once symptomatic, the typical course is further progression with increased neurological impairment. Therefore, once a fistula is diagnosed, treatment is recommended. METHOD: The fistula is placed at the level of intervertebral foramen and surgical ligation is performed through a laminectomy. After dural opening, the area is inspected, and the arterialized vein is identified and ligated. CONCLUSIONS: Laminectomy and arteriovenous fistula ligation is a safe and reliable approach for accessing and treating spinal dural arteriovenous fistulas.

A novel, non-adhesive, precipitating liquid embolic implant with intrinsic radiopacity: feasibility and safety animal study.

OBJECTIVES: To evaluate a novel, ready-to-use, iodinated polyvinyl alcohol polymer embolic implant. METHODS: Under good laboratory practice conditions, 26 pigs were investigated. A complex arteriovenous malformation (AVM) model was created in 16 animals, and a simple rete model was used in the remaining 10 animals. The novel material was used for embolization in 22 animals, and a commercially available liquid embolic material in 4 animals as a control group. Animals were killed at 2 days, 3 months and 6 months. Feasibility, efficacy and safety were evaluated radiologically, clinically and histologically. RESULTS: Preparation was easy, without risk of catheter clogging or adhesiveness. Embolic delivery was well controlled under subtracted fluoroscopy. Visibility was homogeneous throughout the injection and the material behaved cohesively upon delivery. Best lesion penetration was obtained with the use of proximal microballoon occlusion. Unforeseen over-dilution of the test material by DMSO prefilled in the microballoon hub changed the material properties and caused inadvertent cerebral embolization leading to death in five animals. This phenomenon was avoided by practical measures. The casts produced no beam-hardening artefacts on CT scans. Histology showed excellent biocompatibility. CONCLUSIONS: Embolization with this novel, iodinated, precipitating polymer was feasible and effective. Care should be taken during delivery to avoid over-dilution of the material by prefilled DMSO. The material is promising for embolization of AVMs and hypervascular lesions. KEY POINTS: • The intrinsically opaque precipitating polymer has adequate fluoroscopic visibility • The polymer does not induce shading or beam-hardening artefacts on CT • The novel liquid embolic material does not require lengthy preparation • Lack of implant adherence reduces the risk of entrapment of the delivery catheter.

[Spinal dural arteriovenous fistulas. A series of clinical cases and an analysis of the literature data]. / Dural'nye spinal'nye arteriovenoznye fistuly. Seriia sobstvennykh klinicheskikh nabliudenii i analiz dannykh literatury.

Spinal dural arteriovenous fistulas (SDAVFs) are the most common vascular malformation of the spinal cord, causing segmental lesions of the spinal cord due to venous ischemia. Functional outcomes of treatment in SDAVF patients are favorable, but the rate of improvement varies from 25 to 100%, which complicates prediction of the treatment outcome. AIM: the study aim was to identify a relationship between fistula localization and clinical manifestations and evaluate the effect of disease duration and severity of neurological impairments on immediate and long-term treatment outcomes, based on analysis of the literature and own data. MATERIAL AND METHODS: In September 2016, we performed a PubMed search for publications using keywords 'spinal arteriovenous fistula', 'treatment', and 'outcome'. We selected publications containing information on the patient's age, fistula location, disease duration, and evaluation of symptom severity (Aminoff-Logue scale) preoperatively, postoperatively, and at least 3 months after surgery. The analysis also included data on patients operated on at the clinic. A total of 187 patients were included in the analysis. RESULTS: The fistula was most often located at the T6, T7, and T9 level, with motor disorders being more severe for fistulas located at or below the T9 vertebra. Surgical isolation of the fistula improved the functional state of patients, with patients under the age of 60 years having a better prognosis for recovery of impaired functions. Motor disorders significantly regressed in the early postoperative period in all patients, but in the long-term period, there was worsening of motor disorders in patients with a better baseline functional state.

A spectrum of intracranial vascular high-flow arteriovenous shunts in RASA1 mutations.

PURPOSE AND BACKGROUND: We describe three paediatric cases with different intracranial fast-flow shunts presenting early in life, all with capillary malformation-arteriovenous malformation syndrome and RASA1 verified mutations. Intracranial arteriovenous fast-flow shunts are rare vascular malformations typically presenting early in life and have been associated with cutaneous capillary malformations, characterized as capillary malformation-arteriovenous malformation syndrome. Heterozygous RASA1 gene mutations have been found to be disease causing with high penetrance for the typical cutaneous findings, but only some individuals with the syndrome have intracranial lesions. CASES: One infant presented with a vein of Galen malformation responsible for hydrodynamic disorders, one neonate suffered from severe cardiac insufficiency related to a superior sagittal sinus dural malformation with high-flow fistulas, and one baby was treated at infant age of a choroidal arteriovenous fistula discovered antenatally. RESULTS AND CONCLUSIONS: We report the follow-up of these three cases with RASA1 gene mutation and comment on the possible role of evaluation for vascular lesions and capillary malformation-arteriovenous malformation syndrome in patients and their families, with intracranial fast-flow shunts.

Combined radiological and surgical management of spinal dural fistulas.

INTRODUCTION: Spinal Dural Fistulas (SDAVF) are the most common Spinal Vascular Malformation. The management of SDAVFs involves diagnosis and localisation with MRI and spinal angiography and subsequent embolisation or surgical interruption. We report our experience in treating these fistulas in which we undertake endovascular treatment, if technically feasible, with recourse to surgery only if occlusion is not possible. METHOD: This retrospective study reviews the management of 38 patients treated for SDAVFs over a 13-year period (1997-2010). Patient demographics, procedure and follow-up over a 6-12-month period (mean of approximately 10 months) were analysed and a qualitative assessment of patient outcome was made. RESULTS: Patient ages ranged from 20 to 86 (mean 63.5), with 27 male and 11 female patients. A total of 28 patients managed with a combination of embolisation and surgery report either with improved symptoms or a return to normal. The presence of bladder and bowel symptoms indicated more severe disability and their presence at presentation was associated with lack of improvement following treatment. CONCLUSIONS: We report a treatment strategy for SDAVF involving primary endovascular intervention and reserving surgery for those patients in whom endovascular treatment is not possible.

Venous Anatomy of the Central Nervous System.

Comprehensive understanding of venous anatomy is a key factor in the approach to a multitude of conditions. Moreover, the venous system has become the center of attention as a new frontier for treatment of diseases such as idiopathic intracranial hypertension (IIH), arteriovenous malformation (AVM), pulsatile tinnitus, hydrocephalus, and cerebrospinal fluid (CSF) venous fistulas. Its knowledge is ever more an essential requirement of the modern brain physician. In this article, the authors explore the descriptive and functional anatomy of the venous system of the CNS in 5 subsections: embryology, dural sinuses, cortical veins, deep veins, and spinal veins.

Diagnostic, clinical management, and outcomes in patients with spinal dural arteriovenous fistula.

Background: Spinal dural arteriovenous fistulas (SDAVFs) are rare spinal vascular malformations, but account for 70 to 80% of all spinal arteriovenous malformations. SDAVFs can be treated either surgically or endovascularly, with surgical treatment appearing to lead to higher closure rates. Our aim was to analyze the demographic data, diagnostic history, treatment characteristics and clinical short- and long-term outcomes. Methods: The medical records of 81 patients who underwent surgical (n = 70, 86.4%) and endovascular (n = 11, 13.6%) treatment for SDAVF at a university hospital between 2002 and 2023 were retrospectively analyzed. Results: SDAVF was observed more frequently in men than women (61, 75.3% vs. 20, 24.7%) with a mean age of 63.5 ± 12.7 years and a mean duration of symptoms to diagnosis of 12.0 ± 12.8 months. The most common first symptom was gait disturbance (36, 44.4%), followed by sensory disturbance (24, 29.6%). The location of the fistula point was most common in the lower thoracic region (36, 44.5%), followed by the lumbar region (23, 28.4%). Incomplete or failed occlusion of the fistula occurred in 8 patients (9.9%), with 6 patients (7.4%) undergoing further treatment either surgically or endovascularly. Treatment- or hospital-related complications were observed in 16 patients (19.8%). A single-level laminectomy was the most common approach (31, 44.3%), followed by single-level hemilaminectomy (28, 40.0%), and unilateral interlaminar fenestration (11, 15.7%). Back pain or radiculopathy was observed in 58% of patients (47/81) pre-treatment and had already decreased to 24.7% at hospital discharge (p < 0.001). No significant differences were observed in sensory disturbances (p = 0.681). The median of American Spinal Injury Association motor score (ASIA-MS) was 94 [82.5-100] at admission, 98 [86.5-100] at hospital discharge, 100 [90-100] at the first, second, and third follow-up (p = 0.019). The median modified Aminoff-Logue scale (mALS) was 5 [2-7] at admission, 3 [1-6] at hospital discharge, 2 [1-5] at the first follow-up, 2 [0.5-5] at the second follow-up and 2 [1-7] at the third follow-up (p = 0.006). Conclusions: SDAVF occurs predominantly in men in the 6th decade of life and can be safely and effectively treated surgically and endovascularly, improving symptoms such as pain and motor deficits, gait disturbances as well as bowel and bladder dysfunction, but not sensory disturbances.

A Case Series of Late Vascular Lesions of Traumatic Etiology: Endovascular and Surgical Approaches.

There is a broad spectrum of pathology in traumatic vascular injury. Arteriovenous fistula (AVF) is an abnormal communication between the high-flow arterial system and the low-flow venous network, directly connecting the afferent artery and nearby draining veins without the regular intervention of the capillary bed. Most of these fistulas occur due to incidental or iatrogenic injury. A retrospective review of procedures performed by an endovascular surgeon in a tertiary center identified 15 cases of vascular injuries that encompassed all these different clinical scenarios, including post-traumatic, iatrogenic, or spontaneous origin. The information collected, including patient age, sex, previous symptoms, and treatment, was gathered from medical records. In addition, information on procedural technique, endovascular devices used, and specific intraprocedural details were collected from procedure notes and angiographic images. A broad spectrum of injuries can present as late trauma complications (over three months); endovascular treatment is a safe and effective approach for intracranial and extracranial injuries. Endovascular treatment can be a sole option or adjuvant to other hybrid therapies and has emerged as essential for treating these lesions as a first option. We have described standard techniques to treat different vascular pathologies, sometimes with limited resources.

Lumbar Laminectomy Precedes Positional Headache and MRI-Confirmed Pseudomeningocele With Duro-Cutaneous Fistula.

Pseudomeningoceles (PMs) are collections of cerebrospinal fluid (CSF) occurring as a direct result of a dural rent. This article presents a well-documented case of a 68-year-old male presenting to the emergency department with postoperative lumbar PM with a duro-cutaneous fistula. It was initially recognized on palpation of the patient's postoperative incision site and later diagnosed with magnetic resonance imaging (MRI). Incidental durotomies (IDs) leading to PMs are a rare complication of laminectomies and other spinal surgeries. A thorough physical exam, diagnostic imaging, and lumbar drainage to survey the integrity of the dura mater are important aspects of postoperative care.

Endovascular treatment of anterior cranial base dural arteriovenous fistulas as a first-line approach: a multicenter study.

OBJECTIVE: Anterior cranial fossa dural arteriovenous fistulas (DAVFs) have been almost exclusively considered as surgical lesions. However, new advances in endovascular technology have made the endovascular treatment (EVT) of ethmoidal DAVFs feasible. The aim of this study was to report the clinical and angiographic outcomes of patients harboring DAVFs of the anterior cranial fossa who had undergone EVT as a first-line approach. METHODS: This was a retrospective study of a consecutive series of patients harboring anterior cranial base DAVFs who had undergone EVT as a first-line approach at four institutions. Angiographic follow-up was performed at 6 months. Immediate and late serious clinical events were assessed during follow-up, including death and stroke. Special emphasis was given to visual status before and after the treatment. RESULTS: Between 2008 and 2020, 37 patients with ethmoidal DAVFs were admitted to the participating centers. In 2 patients, EVT was not attempted; therefore, 35 patients underwent EVT as a first-line procedure. An isolated transarterial approach was performed in 19 (54.3%) patients. The transvenous approach was performed exclusively in 12 (34.3%) patients, and combined access was used in 4 (11.4%) patients. The most frequently used arterial access route was the ophthalmic artery in 82.6% of the patients. Immediately, complete angiographic occlusion was achieved in 31 (91.2%) of 34 patients whose treatment was accomplished. Six-month control angiography revealed that 30 (88.2%) DAVFs were totally occluded. Complications occurred in 3 (8.8%) patients, including 1 (2.9%) patient who had central retinal artery occlusion. No significant difference in complications or occlusion rates was noted between the transarterial and transvenous approaches. CONCLUSIONS: Most anterior cranial base DAVFs can be successfully treated via an endovascular approach. Neurological and visual complications are rare, even if the ophthalmic artery is used as the primary access route. Efforts should be focused on prospectively comparing the results of EVT and surgical management.

Fatal hemorrhagic complication after coil embolization of a petrosal arteriovenous shunt.

BACKGROUND: Cerebello-pontine AVMs (CPAVMs) and petrous apex dural arteriovenous fistulae (DAVFs) are rare and sometimes difficult to distinguish. We report a fatal hemorrhagic complication after coil embolization of the petrosal vein draining a trigeminal AVM misdiagnosed as a DAVF. CASE PRESENTATION: A 73-year-old woman with a petrous apex arteriovenous shunt with dual dural and pial arterial supply presented with posterior fossa hemorrhage. The draining petrosal vein was catheterized and coiled via the superior petrosal sinus. Two episodes of contrast extravasation occurred during coiling, but the lesion was completely occluded at the end of the procedure. The patient developed a fatal posterior fossa hemorrhage in the recovery room. Microscopic pathology revealed numerous dilated vessels within the trigeminal nerve. CONCLUSION: CPAVMs and DAVFs with pial drainage should be distinguished pre-operatively. Occlusion of a pial vein (as opposed to a sinus) in the treatment of an arteriovenous shunt carries hemorrhagic risk if a liquid embolic agent is not used to completely occlude all pathological vessels.

Cone Beam CT to Guide Transorbital Treatment of a Cavernous Sinus Dural Arteriovenous Fistula in a Patient with Middle Meningeal Artery Origin of the Ophthalmic Artery.

Transorbital puncture to embolize cavernous sinus (CS) dural arteriovenous fistulas (DAVF) is a useful strategy when conventional transvenous routes are inaccessible. We report a case of bilateral CS DAVF associated with bilateral middle meningeal artery (MMA) origin of ophthalmic arteries (OA) who had recently undergone transvenous coil embolization. She presented with persistent symptoms of conjunctival congestion and proptosis in the left eye. Angiogram revealed residual left CS DAVF with dilated SOV. Inferior petrosal sinus or facial vein access was not possible. Transorbital access of the SOV was planned. Cone-beam CT (CBCT) angiography was used to delineate the relationship between the variant OA and SOV and also to plan a safe trajectory. Using fluoroscopy guidance, the SOV was punctured and embolization was done using Onyx-18. CBCT is a valuable tool in planning and executing transorbital treatment of CS DAVF, especially in the setting of variant OA.

The Development of Indirect Carotid Cavernous Fistulas after Microvascular Ischemic 4th Nerve Palsies.

An indirect carotid cavernous fistula (CCF) is an abnormal connection between the cavernous sinus and internal or external carotid artery. Indirect CCFs often occur spontaneously, particularly in the setting of vascular risk factors such as hypertension, diabetes, and atherosclerosis. Microvascular ischemic nerve palsies (NPs) share these vascular risk factors. However, to date, no temporal relationship between microvascular ischemic NP and indirect CCF occurring sequentially has been reported. We describe the cases of 64- and 73-year-old women who developed indirect CCFs within 1-2 weeks after spontaneous resolution of a microvascular ischemic 4th NP. Both patients had complete resolution and an asymptomatic period between the 4th NP and CCF. This case highlights the shared pathophysiology and risk factors between microvascular ischemic NPs and CCFs, and emphasizes that CCFs should be kept in the differential diagnosis for red eye or recurrent diplopia in patients with previous microvascular ischemic NP.

Lateral Variant of Davidoff and Schechter Dural Arteriovenous Fistula.

Dural arteriovenous fistulas represent a distinct direct pathological connection between dural arterial feeders of the meninges to cortical veins or sinuses. Vascular supply of the meninges is provided by a series of named arteries-anterior, middle, and posterior meningeal arteries, with the tentorium provided by the artery of Bernasconi and Cassinari (anterior-medial) and the artery of Davidoff and Schechter (posterior-medial). This case is the first report in the literature of a lateral distal posterior cerebral artery supplying the meninges and contributing dural feeders to a Cognard type III/Borden type III dural arteriovenous fistula.

Microsurgical Treatment of a Complex Cognard V Tentorial Dural Fistula with Superior Cerebellar Artery Supply.

This case video demonstrates a microsurgical technique for the clipping and obliteration of a Cognard V tentorial dural fistula (Video 1). The patient was a 49-year-old male who presented with progressive upper and lower extremity weakness over 12 months, with associated cervical spinal cord edema. The patient was initially misdiagnosed with transverse myelitis; however, abnormal flow voids on magnetic resonance imaging led to a cerebral angiogram being performed. The preoperative angiogram demonstrated the Cognard V right tentorial dural arteriovenous fistula with drainage into the dorsal and ventral medullary veins. The fistula resulted in spinal cord symptoms due to spinal cord venous engorgement, with a lack of cranial symptoms. In these cases, microsurgery is the preferred method of treatment due to excellent surgical window to the medial tentorial margin and difficulty in catheterizing the small tortuous superior cerebellar artery meningeal feeder. This is a novel case showing a hybrid operating room technology to safely approach a complex fistula and obtain curative confirmation by transradial intraoperative angiography. In addition, this case is unique in providing a surgical visualization of the meningeal superior cerebellar artery feeder contributing to this fistula, namely the artery of Wollschlaeger & Wollschlaeger. Postoperatively, the patient demonstrated significant improvement in upper and lower extremity strength, indicative of a successful recovery. The patient was discharged to rehabilitation, with continued motor improvement.